Perigraft hygroma mimicking recurrent angiosarcoma of the right atrium

Primary cardiac angiosarcoma is extremely rare, has a poor prognosis and has no specific clinical manifestation. A 31-year-old pregnant woman presented to the emergency department with signs of pericardial tamponade. Transesophageal echocardiography (TEE) showed a mass within her right atrium. Extensive surgical resection was performed with subsequent pericardial patch reconstruction of the right atrium. Histological examination showed the presence of an angiosarcoma. A reoperation was performed 18 months after the initial operation, because of concerns of radicality. Three years later she was re-admitted with a superior caval vein syndrome. A stent was placed to relieve symptoms. Because of the suspicion of a recurrent tumor, a second reoperation followed. During this operation, a perigraft hygroma was found pressing against the previously reconstructed superior caval vein. Histological examination after both reoperations showed no signs of a recurrent angiosarcoma. Until today, 8 years after her first surgery, the patient is still alive without any signs of recurrent tumor.